Peroxisomes Are Required for Lipid Metabolism and Muscle Function in Drosophila melanogaster

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dc.citation.firstpagee100213
dc.citation.issueNumber6
dc.citation.journalTitlePLoS ONE
dc.citation.volumeNumber9
dc.contributor.authorFaust, Joseph E.
dc.contributor.authorManisundaram, Arvind
dc.contributor.authorIvanova, Pavlina T.
dc.contributor.authorMilne, Stephen B.
dc.contributor.authorSummerville, James B.
dc.contributor.authorBrown, H.Alex
dc.contributor.authorWangler, Michael F.
dc.contributor.authorStern, Michael
dc.contributor.authorMcNew, James A.
dc.date.accessioned2015-07-09T20:14:49Z
dc.date.available2015-07-09T20:14:49Z
dc.date.issued2014
dc.description.abstractPeroxisomes are ubiquitous organelles that perform lipid and reactive oxygen species metabolism. Defects in peroxisome biogenesis cause peroxisome biogenesis disorders (PBDs). The most severe PBD, Zellweger syndrome, is characterized in part by neuronal dysfunction, craniofacial malformations, and low muscle tone (hypotonia). These devastating diseases lack effective therapies and the development of animal models may reveal new drug targets. We have generated Drosophila mutants with impaired peroxisome biogenesis by disrupting the early peroxin gene pex3, which participates in budding of pre-peroxisomes from the ER and peroxisomal membrane protein localization. pex3 deletion mutants lack detectible peroxisomes and die before or during pupariation. At earlier stages of development, larvae lacking Pex3 display reduced size and impaired lipid metabolism. Selective loss of peroxisomes in muscles impairs muscle function and results in flightless animals. Although, hypotonia in PBD patients is thought to be a secondary effect of neuronal dysfunction, our results suggest that peroxisome loss directly affects muscle physiology, possibly by disrupting energy metabolism. Understanding the role of peroxisomes in Drosophila physiology, specifically in muscle cells may reveal novel aspects of PBD etiology.
dc.identifier.citationFaust, Joseph E., Manisundaram, Arvind, Ivanova, Pavlina T., et al.. "Peroxisomes Are Required for Lipid Metabolism and Muscle Function in Drosophila melanogaster." <i>PLoS ONE,</i> 9, no. 6 (2014) Public Library of Science: e100213. http://dx.doi.org/10.1371/journal.pone.0100213.
dc.identifier.doihttp://dx.doi.org/10.1371/journal.pone.0100213
dc.identifier.urihttps://hdl.handle.net/1911/80864
dc.language.isoeng
dc.publisherPublic Library of Science
dc.rightsThis is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
dc.rights.urihttps://creativecommons.org/licenses/by/4.0/
dc.titlePeroxisomes Are Required for Lipid Metabolism and Muscle Function in Drosophila melanogaster
dc.typeJournal article
dc.type.dcmiText
dc.type.publicationpublisher version
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